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Intrauterine myocardial infarction is a rare and frequently fatal diagnosis. It has been presented in the literature only as case reports and short series. We present a case report of a coronary occlusive intrauterine myocardial infarction and survival and present a systematic review of the literature. This is the first summative description of current data on intrauterine and perinatal myocardial infarction. We performed the systematic review based on the guidelines established by the PRISMA statement. Our population of intrauterine and perinatal myocardial infarction included published cases who presented as a live birth within the first 28 postnatal days, and had a diagnosis of myocardial infarction. We conducted descriptive statistics and regression analysis on short-term mortality as the primary outcome. After applying exclusion criteria we described 84 individual cases of myocardial infarction from 63 full-text articles including our own case. Presentation within the first 12 hours was associated with mortality (OR 3.90, p=0.004). Treatment modalities were varied and inconsistently recorded. The aetiologies and comorbidities are varied in our systematic review. We would have a low threshold to perform viral testing, consider anticoagulation early and coronary imaging if feasible. The use of extracorporeal membranous oxygenation may serve as a bridge to cardiac recovery.
Critical heart disease in the pediatric population is associated with high morbidity and mortality. Research around the most effective communication and decision-making strategies is lacking. This systematic review aims to summarise what is known about parent preference for communication and decision-making in children with critical heart disease. Database searches included key words such as family, pediatric heart disease, communication, and decision-making. A total of 10 studies fit our inclusion criteria: nine were qualitative studies with parent interviews and one study was quantitative with a parent survey. We found three main themes regarding physician–parent communication and decision-making in the context of paediatric heart disease: (1) amount, timing, and content of information provided to parents; (2) helpful physician characteristics and communication styles; and (3) reinforcing the support circle for families.
Growth failure is prevalent among infants with CHD. A Standardized Clinical Assessment and Management Plan was introduced at Boston Children’s Hospital’s cardiac medical ward to identify patients with growth failure, evaluate relevant contributing conditions, and recommend a management plan including collaboration with nutrition physicians.
The objective of this study was to determine whether enrolled patients had improved growth compared with historical controls.
A total of 29 patients were enrolled in the period July, 2013–June, 2014. In all, 42 historical controls who met eligibility criteria for enrolment were selected for comparison from patients admitted to the same ward in the period June, 2010–June, 2011. Patients with CHD aged <1 year , with growth failure defined as weight-for-age z-score <−2, or failure to sustain adequate weight gain were eligible for participation. Primary outcome was change in weight-for-age z-score from enrolment to most recent weight measurement among patients with at least 6 months of follow-up.
Control patients were older at baseline admission weight (118 versus 95 days, p=0.33), and had a higher weight-for-age z-score, −2.9 (−3.1, −2.6) versus −3.7 (−4.3, −3.0) (p=0.02), compared with enrolled patients. Enrolled patients had greater gain in weight-for-age z-score, 2.7 (2.0, 3.4) versus 1.8 (1.5, 2.2) (p=0.03), from baseline to most recent follow-up.
Patients enrolled in a nutrition-focused protocol had greater weight improvement than historical controls. Identification of growth failure and collaboration with a nutrition support team was associated with improved weight gain among CHD patients experiencing growth failure. CHD programmes should consider a structural approach, including nutrition expertise to address growth failure.
Anecdotal reports suggest that children and young adults with CHD frequently experience pain in their legs. The purpose of this pilot study, performed by Little Hearts Matter patient organisation, was to assess the burden of leg pains in this group and begin to investigate associated factors and consequences for daily living.
An internet-based survey was distributed by Little Hearts Matter patient organisation. After anonymisation and collation, responses were analysed and compared with their healthy siblings.
Of the 220 patients who responded, 94% reported leg pains compared with 30% of siblings (n=107; p<0.001). In respondents, pain was typically reported to occur in the lower legs or around the knees or ankles, often associated with crying and screaming (49.0%) and most commonly occurring at night-time (82.0%). Individuals taking aspirin and those who were more active were more likely to report leg pains. Older age was associated with leg pain that occurred with stress (p=0.02) and at night (p=0.05). Analgesia (64.1%) or massage (53.9%) was the preferred option for alleviation. There was no gender bias, association with diagnosis, surgical history, and/ or relationship with diagnosed orthopaedic issues.
Leg pains are more frequent in those with CHD compared with their healthy siblings. Aetiology is uncertain, but pains share many common characteristics with benign “growing pains”.
Infantile haemangioma is one of the most commonly known benign vascular tumours of infancy and childhood, having an incidence of 3–10%. Most lesions regress spontaneously; however, some may require treatment owing to their clinical and cosmetic effects. Propranolol has become the treatment of choice for infantile haemangioma, but treatment protocols are largely institutional based without any specific consensus guidelines. Our aim was to evaluate the cost-effectiveness of propranolol use as inpatient versus outpatient therapy.
A decision tree model was created depicting alternate strategies for initiating propranolol treatment on an inpatient versus outpatient basis combined with the option of a pretreatment echocardiogram applied to both strategies. Cost analysis was assumed to be based on treatment of haemangioma in patients who were born at term, had no chronic illnesses, a non-life-threatening location of the haemangioma, and those who were not taking any other medications that could potentiate the side effects of propranolol. A sensitivity analysis was performed to evaluate the probability of side effects.
The average cost incurred for inpatient treatment of infantile haemangioma was approximately $2603 for a single hospital day and increased to $2843 with the addition of an echocardiogram. The expected cost of treatment in the outpatient setting was $138, which increased to $828 after the addition of an echocardiogram.
Treating infantile haemangioma with propranolol is more cost-effective when initiated on an outpatient basis.
The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement.
Prospectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified.
Preoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9–5.7) and 5.7 kg (5.5–6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5–100%). Digoxin was used by 11/14 centres in 25% of patients (23–31%), and 81% had some oral feeds (68–84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75–113). In aggregate, 26% of patients had deep hypothermic circulatory arrest >10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8–32%). Seven centres extubated 5% of patients (2–40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0–5.3) and total length of stay was 7.5 days (6–10).
In the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.
Static balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD.
We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect.
The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases.
Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect.
The outcome of transcatheter closure in ostium secundum atrial septal defects is determined by the morphology of the defects. Modified techniques such as balloon assistance, pulmonary vein deployment, left atrial roof technique, and so on are used for circumventing the anatomic complexities and increasing the success rates.
We planned a prospective study looking at the outcomes of transcatheter closure in secundum atrial septal defects with modified techniques in different anatomic complexities identified in transoesophageal echocardiography and their association with outcome of transcatheter closure.
Transcatheter closure was successful in 295 out of 346 (82%) patients with modified techniques. Balloon-assisted technique offered a success rate of 87%. The mean defect size was 34.7±2.78 mm (95% confidence interval (CI) 30.67–43.1 mm) with success and 40.16±4.5 mm (95% CI 32.16–44.7) with failure (p = 0.02). The mean total septal length was 38.11±0.63 (95% CI 35.21–40.56 mm) with success and 42.54±0.34 (95% CI 38.79–43.21 mm) with failure. The defect to septal ratios were 0.82 and 0.94 in success and failure groups, respectively (p=0.02). However, the absence of a retro-aortic margin, septal aneurysm, and multiple defects did not affect the success rate. Deficient inferior vena caval margin, deficient posterior margin, and size⩾40 mm had a high risk of failure with transcatheter closure. The odds ratio for procedural failure was 25.3 (4.3–143.8) in patients with malaligned septum, 8.3(1.4–48.5) with deficient inferior vena caval margin, and 4.1(2.5–19) for size⩾40 mm.
The modified techniques for device deployment offer substantial chances of success in transcatheter closure of secundum atrial septal defects with anatomical complexity (82%). Variants such as defect size of⩾40 mm and deficient inferior and posterior margins have high failure rates with a modified technique.
Traditional ambulatory rhythm monitoring in children can have limitations, including cumbersome leads and limited monitoring duration. The ZioTM patch ambulatory monitor is a small, adhesive, single-channel rhythm monitor that can be worn up to 2 weeks. In this study, we present a retrospective cross-sectional analysis of the ZioTM monitor’s impact in clinical practice. Patients aged 0–18 years were included in the study. A total of 373 studies were reviewed in 332 patients. In all, 28.4% had structural heart disease, and 16.9% had a prior surgical, catheterisation, or electrophysiology procedure. The most common indication for monitoring was tachypalpitations (41%); 93.5% of these patients had their symptoms captured during the study window. The median duration of monitoring was 5 days. Overall, 5.1% of ZioTM monitoring identified arrhythmias requiring new intervention or increased medical management; 4.0% identified arrhythmias requiring increased clinical surveillance. The remainder had either normal-variant rhythm or minor rhythm findings requiring no change in management. For patients with tachypalpitations and no structural heart disease, 13.2% had pathological arrhythmias, but 72.9% had normal-variant rhythm during symptoms, allowing discharge from cardiology care. Notably, for patients with findings requiring intervention or increased surveillance, 56% had findings first identified beyond 24 hours, and only 62% were patient-triggered findings. Seven studies (1.9%) were associated with complications or patient intolerance. The ZioTM is a well-tolerated device that may improve what traditional Holter and event monitoring would detect in paediatric cardiology patients. This study shows a positive clinical impact on the management of patients within a paediatric cardiology practice.
We currently perform open-heart procedures using bloodless priming of cardiopulmonary bypass circuits regardless of a patient’s body weight. This study presents results of this blood-saving approach in neonates and infants with a body weight of up to 7 kg. It tests with multivariate analysis factors that affect perioperative transfusion. A total of 498 open-heart procedures were carried out in the period 2014–2016 and were analysed. Priming volume ranged from 73 ml for patients weighing up to 2.5 kg to 110 ml for those weighing over 5 kg. Transfusion threshold during cardiopulmonary bypass was 8 g/dl of haemoglobin concentration. Transfusion factors were first analysed individually. Variables with a p-value lower than 0.2 underwent logistic regression. Extracorporeal circulation was conducted without transfusion of blood in 335 procedures – that is, 67% of cases. Transfusion-free operation was achieved in 136 patients (27%) and was more frequently observed after arterial switch operation and ventricular septal defect repair (12/18=66.7%). It was never observed after Norwood procedure (0/33=0%). Lower mortality score (p=0.001), anaesthesia provided by a certain physician (p=0.006), first chest entry (p=0.013), and higher haemoglobin concentration before going on bypass (p=0.013) supported transfusion-free operation. Early postoperative mortality was 4.4% (22/498). It was lower than expected (6.4%: 32/498). In conclusion, by adjusting the circuit, cardiopulmonary bypass could be conducted without donor blood in majority of patients, regardless of body weight. Transfusion-free open-heart surgery in neonates and infants requires team cooperation. It was more often achieved in procedures with lower mortality score.
There is no standard dose or protocol for beta-blocker administration as preconditioning in children undergoing coronary CT angiography.
A total of 63 consecutive patients, with a mean age of 10.0±3.1 years, who underwent coronary CT angiography to assess possible coronary complications were enrolled in a single-centre, retrospective study. All patients were given an oral beta-blocker 1 hour before coronary CT angiography. Additional oral beta-blocker or intravenous beta-blocker was given to those with a high heart rate. We compared image quality, radiation exposure, and adverse events among the patients without additional beta-blocker, with additional oral beta-blocker, and with additional intravenous beta-blocker.
There were no significant differences in image quality or radiation exposure among the groups. The heart rate just before scanning was significantly correlated with image quality (p<0.001, r=−0.533) but was not correlated with radiation exposure (p=0.45, r=0.096). There were no adverse events related to any allergic reaction, thereby showing the effectiveness of the beta-blocker.
Initial oral beta-blocker administration (0.8 mg/kg/dose) should be administered to all children undergoing coronary CT angiography. Additional intravenous beta-blocker should be given to those with poor heart rate control to improve image quality without increasing radiation exposure or allowing adverse events.
Communications between the pulmonary artery and left atrium cause cyanosis. The images document serial haemodynamic changes in such a fistula from fetal life to the postnatal period with a successful transcatheter intervention.
Left ventricular assist device implantation is an important therapeutic option for children with end-stage heart failure. However, device-related complications such as infection may occur while the patient is supported. Device-associated infection can be life-threatening, and early detection is critical. F-fluorodeoxyglucose positron emission tomography and CT is a highly sensitive imaging modality for the detection of an inflammatory response and is useful to evaluate the response to antibiotic therapy. We present two case reports of a left ventricular assist device-associated infection detected by F-fluorodeoxyglucose positron emission tomography and CT in children.
Hydatid disease is a zoonotic parasitic infection endemic in livestock-raising countries. Isolated cardiac hydatid cyst is a very rare disease, and chest pain, palpitations, cough, and dyspnoea are the most common presenting symptoms. Here a case of isolated cardiac hydatid cyst in a female patient with chest pain and electrocardiographic changes mimicking myocardial ischaemia is presented.
Only few reports have described successful simultaneous transcatheter intervention for CHD in infants. We present an infant with secundum atrial septal defect complicated by valvular pulmonary stenosis. Percutaneous transcatheter pulmonary valvuloplasty was performed first, followed by transcatheter closure of the secundum atrial septal defect uneventfully. Simultaneous transcatheter correction is an effective therapeutic option even in infants.
Kounis Syndrome is characterised by the concurrence of acute coronary syndrome with mast cell activation induced by inflammatory mediators released during an allergic reaction. Although several factors and diseases were reported to be associated with Kounis Syndrome, branched-chain amino acid supplements have not been previously reported as a cause of Kounis Syndrome. We present a 17-year-old boy admitted to our hospital with thoracic pain after the ingestion of a branched-chain amino acid supplement.
Percutaneous pulmonary valve implantation is increasingly adopted as an alternative procedure to surgery in dysfunctional homograft, and in patients with “native” or wide right ventricle outflow tract dysfunction. Pre-stenting is mandatory in this category of patients for many reasons, one of which is to create an adequate landing zone for the bioprosthesis. Here we report on a tricky situation that occurred during pre-stenting, and we describe how we successfully overcame it.
Scimitar or pulmonary venolobar syndrome, a rare pulmonary anomaly, consists basically of anomalous pulmonary venous drainage of the right lung to the inferior caval vein, anomalous systemic arterial supply to the right lower lobe from the descending aorta, hypoplasia of the right lung, and dextroposed heart. We present a rare case with constellation of all these findings of scimitar syndrome, but with the aberrant pulmonary vein draining into the left atrium.